LAM mainly affects the lungs, where it causes an overgrowth of a smooth muscle cell.
This overgrowth occurs around the airways (breathing tubes) and also around the blood vessels and the lymph vessels (lymph vessels normally drain excess fluid from the lungs). As the smooth muscle cell proliferates the lung tissue is overgrown and replaced by thin walled cysts, sometimes called “blebs”.
The main effects of LAM are in the lung, however some patients will develop angiolymphomas (benign tumours in the kidney) Lymph nodes may be enlarged in other parts of the body, occasionally LAM will present in the abdomen and some women experience problems with chyle, a milky white fluid build up. Chyle may present in the tummy and chest.
The name lymphangioleiomyomatosis reflects the different components of the disease. Lymph and angio refer to the lymph and blood vessels that are involved and leiomyo refers to the smooth muscle. LAM is sometimes called sporadic LAM to distinguish it from LAM that occurs in patients who have tuberous sclerosis.
